Operative management of intestinal atresia and stenosis based on pathologic findings

Operative Management of Intestinal Atresia and Stenosis Based on Pathologic Findings By Jay L. Grosfeld, Thomas V. N. Ballantine, and Robert Shoemaker Indianapolis, Indiana 9 This report describes the individualized operative therapy in 75 patients with intestinal atresia or stenosis based on pathological findings ( 1 9 7 2 1978). Location of obstruction was duodenal in 30, jejunoileal in 38, and colonic in 7. Duodenal atresia was noted in 2 3 infants and stenosis in 7. Associated anomalies w e r e observed in 23 patients, prematurity in 11, polyhydramnios in 11, and Down's Syndrome in 10. Operative treatment included duodenoduodenostomy (DD)in 19 patients, duodenotomy and web excision in 9, and side-toside duodenojejunostomy (DJ) in 2. Both (D J) cases needed revision to (DD) because of obstruction. A gastrostomy was used in all cases. Jejunoileal atresia occurred in 33 patients and stenosis in 5. Operations included wide proximal resection and endto-end anastomosis in 18 patients with normal bowel length, minimal resection with antimesenteric tapering enteroplasty (using autostaples) and anastomosis in 9 patients with foreshortened bowel, and resection and double barrel enterostomy in 6 with peritonitis or questionable bowel viability. Seventy percent received total parenteral nutrition (TPN). Six patients with colon atresia and one infant with stricture of the rectosigmoid survived initial colostomy and subsequent anastomosis at 3 to 6 mo of age. Survival for duodenal cases was 8 4 % , jejunoileal 90 % , and colonic 1 0 0 % . Individualizing the operative management according to pathologic findings and TPN support when indicated improve operative survival in infants with intestinal atresia. INDEX WORDS: Duodenal atresia; jejunoileal atresia; colonic atresia; tapering enteroplasty.

LIMENTARY T R A C T o b s t r u c t i o n in t h e n e w b o r n i n f a n t m a y be d u e to a v a r i e t y of a n o m a l o u s conditions, a m o n g t h e m a t r e s i a a n d stenosis o f t h e intestine. D u r i n g the p a s t d e c a d e , r e f i n e m e n t s in o p e r a t i v e a n d a n e s t h e t i c tech-

A

From the Section of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine and The James Whitcomb Riley Hospitalfor Children, Indianapolis, Ind. Presented before the 27th Annual Meeting of the Surgical Section of the American Academy of Pediatrics, Chicago, Illinois, October 22-23, 1978. Address reprint requests to Jay L. Grosfeld, M.D., J. W. Riley Hospital for Children, Rm. K-21, I100 West Michigan Street, Indianapolis, Ind. 46223. 9 1979 by Grune & Stratton, Inc. 0022-3468/79/1403-0030501.00/0 368

n i q u e s a n d pre- and p o s t o p e r a t i v e c a r e (especially in the a r e a o f n u t r i t i o n a l s u p p o r t ) has led to an i m p r o v e m e n t in survival. T h i s r e p o r t d e s c r i b e s t h e c u r r e n t c o n c e p t s of t r e a t m e n t a n d a favorable experience using individualized opera t i v e t h e r a p y b a s e d on p a t h o l o g i c a l findings. PATIENT MATERIAL Seventy-five infants with intestinal atresia and stenosis were treated at the James Whitcomb Riley Hospital for Children on the Indiana University Medical Center Campus from July, 1972 to July, 1978. The location of obstruction was duodenal in 30 patients, jejunoileal in 38, and colonic in 7.

Duodenal Atresia and Stenosis Duodenal obstruction was observed in 30 patients, 16 of whom were girls, and 14 of whom were boys. Maternal polyhydramnios was observed in I I cases, prematurity in I 1, and Down's Syndrome in 10 patients. Pertinent clinical findings included bilious vomiting in 27 patients and upper abdominal distention in 21. Associated congenital anomalies were observed in 23 patients (Table 1). In each instance, diagnosis was achieved by plain abdominal radiographs that demonstrated the classical "double bubble" sign characteristic of duodenal obstruction. Contraststudies were useful in instances of duodenal stenosis with partial obstruction. Preoperative preparation was frequently required because of hypovolemia and electrolyte imbalance. Operation was performed in each instance through a right supraumbilical transverse incision. At operation, obstruction was related to atresia in 23 cases and stenosis in 7. A duodenal web was present in 9 cases, annular pancreas in 5, malrotation in 12, and an anterior portal vein in 1. The duodenal wall was in continuity in 27 cases and the ends separated in 3. Operations included duodenotomy and web excision in 9 patients with a duodenal web, duodenoduodenostomy in 19 patients, and side-to-side duodenojejunostomy in two. Stamm gastrostomy was performed as an adjunctive procedure in each case and a Ladd procedure was done in those patients with malrotation. Following mobilization of the duodenum, identification of duodenal web was accomplished by intragastric insertion of a red rubber catheter through a gastrotomy site which was then passed distally to identify the site of obstruction. The site was usually indicated by a slight constriction of the duodenal wall. Duodenal webs were attached peripherally at the ampullary level. The ampulla of vater was carefully identified in cases treated by direct duodenal operation by carefully compressing the gall bladder and observing the location of bile flow. The two patients with duodenojejunostomy required revision to duodenoduodenostomy due to late obstruction. No Journal of Pediatric Surgery, Vol. 14, No. 3 (June), 1979

369

INTESTINAL ATRESIA AND STENOSIS

Table 1. Duodenal Atresia Associated Anomalies * 2 3 / 3 0 (76.6 % ) Type

No. of P a t i e n t s

Percent

Cardiac

16

53.3

Malrotation

12

40.0

Annular pancreas

5

16.6

TE-fistula Imperforate anus

3 3

1O.O 10.O

Jejunal atresia

1

3.3

Anterior portal vein

1

3.3

Intestinal atresia

1

3.3

Hydrocephalus

1

3.3

Associated anomalies occurred in over 75% of patients with duodenal common.

obstruction.

Cardiovascular

defects

were

most

significant complications were referrable to the duodenotomy or duodenoduodenostomy procedures. The operative mortality was 3.5% ( l / 3 0 ) - - t h i s single death occurred in a 3-1b premature infant with respiratory distress and sepsis. There were 4 late deaths, due to associated anomalies in 1 patient (age 6 mo), a crib death in a 5 mo old, and in 2 infants with Down's Syndrome due to pneumonia. The overall survival was 84% (25/30).

Jejunoileal Atresia and Stenosis Jejunoileal atresia and stenosis was observed in 38 patients. Thirty-three had small bowel atresia while five had stenosis. There were 16 boys and 22 girls. Thirty-five infants were white and three were black. Eight of the infants were premature, one had cystic fibrosis, and one had Down's Syndrome. Five cases occurred in neonates with gastroschisis. Polyhydramnios and jaundice were more common in instances of jejunal obstruction, while abdominal distention was observed more frequently in cases involving the ileum (Fig. 1). Erect and recumbent abdominal radiographs frequently showed a few distended loops of intestine in jejunal obstructions and often demonstrated many dilated loops of intestine with air-fluid levels in cases of ileal obstrucJEJUNO-ILEAL ATRESIA

&

STENOSIS:CLINICAL

tion. Barium enema was done in each instance and showed a complete microcolon (unused colon) locating the level of obstruction to the small bowel. At operation, atresia involved the ileum in 18 cases and the jejunum in 15, while stenosis occurred in the jejunum in 3 and the ileum in 2. Atresias were classified as Type I (mucosal), 2 cases, Type II (band), 7 cases, Type llla (V-shaped gap), 14 cases, Type lllb ("apple peel"), 4 cases, and Type tV (multiple), 6 cases (Fig. 2). The operation of choice in instances of jejunoileal atresia was related to the pathological findings and the specific set of circumstances encountered in each case. The actual decision as to the most appropriate procedure was dependent upon the type and location of atresia and a number of variables including the presence of malrotation, gastroschisis, volvulus, meconium ileus, peritonitis, and short gut. Twelve patients had short gut that was defined as those infants presenting with less than one-half of their estimated bowel length using 200 cm as "normal." Eight patients had malrotation, twelve, peritonitis, and sixteen, evidence of intrauterine volvulus. Operative treatment included wide proximal resection and end-to-end anastomosis in 18 cases (10 jejunal, 8 ileal) and minimal resection with antimesenteric tapering enteroplasty and end-to-end anastomosis in 9 (5 jejunal, 4 ileal) (Fig. 3A-C). Wide proximal resection was employed in patients with adequate length of bowel while the latter procedures were usually done in certain patients with short gut and those with generalized distention of the remaining proximal small bowel. In addition, six infants with ileal atresia and severe peritonitis or questionable bowel viability underwent resection and formation of a temporary side-by-side double barrel enterostomy. These included 3 infants with gastroschisis and 2 with short gut.

FINDINGS

["~JEJUNAL

, POLYHYD/~AMNIOS

~

BILIOUS VOMITING

~.~jjjjjjjj~.S~jjjjjjj~

I

F T ~ ~LEAL

A

1

I ABDOMINAL DISTENTION

i

i

FAILURE TO PASS MECONIUM

;/ ~ J J J J J J J ~ ~ J J J J J J ~ J J,J J J J J ~

JAUNDICE

I

I

20

40

I 60 PERCENT

I

l

80

100

OF CASES

Fig. 1. Bar graph demonstrating that polyhydramnios and jaundice occurred more frequently with jejunal obstruction while abdominal distention was more common with ileal obstruction.

Fig. 2. Suggested classification of jejunoileal atresia: Type I--mucosal, Type U--atretic ends separated by fibrous cord, Type Illa--atretic ends separated by Vshaped mesenteric gap, Type IIIb--"apple peel" atresia, Type IV--multiple atresias.

370

GROSFELD, BALLANTINE, AND SHOEMAKER

~4x90"

B ~---_~J~

A I. j.

-

,

1

.;

BENSON

JEJUNAL ATRESIA

5

i

Fig. 3. (A) (a) Patients with jejunal atresia responded satisfactorily to wide proximal resection back to the ligament of Treitz and (b) A 9 0 ~ to 4 5 ~ end-to-oblique anastomosis. (b) In instances of jejunal atresia with short bowel, tapering enteroplasty over a No. 2 2 - 2 4 F catheter using staples was beneficial. (C) W i d e proximal resection and anastomosis was a useful procedure in infants with ileal atresia and relatively normal bowel length.

A 90 ~ proximal to 45 ~ distal end-to-oblique anastomosis was fashioned using the techniques of Benson and Nixon (Fig. 3A). ~5'~6Two layers of interrupted atraumatic 5-0 silk were used. Tapering enteroplasty was accomplished by resection of the antimesenteric segment of the dilated proximal bowel over a No. 22-24 F red rubber catheter to assure an adequate lumen size. This was facilitated by using an autostapling instrument. Bleeding points along the staple line were cauterized by a fine tip infant electrocoagulator and the staple line was inverted with a row of interrupted 5-0 silk sutures (Fig. 3B). Formation of the enterostomy was accomplished by securing the bowel to the peritoneum and fascia with a few interrupted fine 5-0 silk seromuscular sutures to prevent stomal retraction as well as peristomal hernia and evisceration. The procedures were done using a fiberoptic headlight source and magnifying loupes (2.5-3.0X) that made the anastomotic techniques more precise.

Four of the patients with jejunal atresia were initially operated upon elsewhere. Three had an end-to-end anastomosis without resection of the proximal limb while one had a side-to-side anastomosis without resection. All four patients were transferred with persistent obstruction and underwent successful resection and anastomosis. The six patients with temporary enterostomies subsequently had successful closure by end-to-end anastomosis at 3 mo of age. Five patients with stenosis of the small intestine were managed by resection and primary end-to-end anastomosis using the above mentioned techniques. This included two patients with gastroschisis. In one, a jejunal stenosis was observed and managed by excision of the stenotic web and an enteroplasty, while in the second case, an ileal web was missed at early attempts at abdominal wall closure and was noted at reexploration for intestinal obstruction. This was managed by resection and anastomosis. Adjunctive gastrostomy was usually employed in patients with jejunal atresias,

INTESTINAL ATREStA AND STENOSIS

those with "short gut," and in infants with peritonitis and formation of enterostomy exclusive of gastroschisis patients. During the postoperative period, delays in bowel function or diarrhea related to "short bowel" necessitated the use of total parenteral nutrition through a tunnelled central venous catheter in 26 of 38 patients (70%). When appropriate bowel function was demonstrated and the nasogastric or gastrostomy returns were clear and of low volume, oral or gastrostomy feedings were initiated. A small curd, non-lactose formula (Nutramigen, Isomil, Pregestimil) and in cases of short gut, Portagen (a medium chain triglyceride diet) or an elemental diet such as unflavored Vivonex were offered. Efforts were made to insure an intake of 120/cal/kg/day by combined oral and intravenous alimentation, gradually weaning the infant from intravenous alimentation (peripheral or total) when full oral intake was tolerated. During the postoperative period, all infants with jejunoileal atresia were evaluated for cystic fibrosis by undergoing a sweat chloride determination. In only one instance was this positive. Significant postoperative complications included 3 instances of pneumonia, 1 wound infection, and 1 case of intestinal infarction due to volvulus. The latter case was managed by massive resection of infarcted tissues and temporary enterostomy. This patient had only 20 cm of proximal jejunum remaining. Enterostomy was closed at 3 too. After 14 mo of total parenteral nutrition, the patient was discharged on a full diet. Eight additional patients with "short gut" having from 30 85 cm of remaining bowel also survived. In addition, all 6 patients with multiple atresias, 3 of 4 with "apple peel" atresias, 4 of 5 with gastroschisis, and 10 of 12 with meconium peritonitis survived. There were no anastomotic leaks observed. The operative mortality (first 30 days) was zero. There were four late deaths (4/38 10.5% mortality). A premature infant with gastroschisis and ileal atresia died at age 3 mo of respiratory insufficiency and bronchopulmonary dysplasia. Three additional infants with short bowel, ages 6, 7, and 8 too, respectively, died of sepsis while receiving total parenteral nutrition. One had an "'apple peel" atresia and two initially had meconium peritonitis.

Colonic Atresia and Stenosis Six patients had colonic atresia, involving the transverse colon in five cases and the sigmoid colon in one. Four patients were girls and two were boys. All were full-term babies, five weighed more than 3 kg and one had gastroschisis and was small for gestational age. Only one of these patients had associated musculoskeletal anomalies. Five patients demonstrated a Type Ilia (gap) atresia, while one had a Type 1 (mucosal) atresia. Five patients presented with abdominal distention, bilious vomiting, and failure to pass meconium in the first 24 hr of life. Plain abdominal radiographs showed dilated intestine with one very large loop containing an air-fluid level or air mixed with meconium ("soap-bubble" sign). Barium enema demonstrated a blind-ending unused distal colon. The colon atresia in the patient with gastroschisis was missed at an initial operation at another institution. Following transfer, the atresia was observed at a subsequent laparotomy for intestinal obstruction. One patient with a stricture of the sigmoid colon presented with abdominal distention and bilious vomiting at 2 days of age. Barium

371

enema showed the stricture at the rectosigmoid level. All 7 patients (100%) survived initial end-colostomy and subsequent anastomosis at 3-6 mo of age. Two patients had subsequent reoperation, however, for adhesive bowel obstruction, but tolerated appropriate enterolysis satisfactorily.

DISCUSSION T h e etiology of intestinal atresia m a y v a r y a c c o r d i n g to t h e site o f o c c u r r e n c e . For e x a m p l e , m a n y i n s t a n c e s of d u o d e n a l atresia a r e p r o b a b l y r e l a t e d to a l a c k o f r e v a c u o l i z a t i o n o f the solid c o r d s t a g e of intestinal d e v e l o p m e n t as p r o p o s e d by T a n d l e r in 1900 and c o n f i r m e d , to s o m e d e g r e e , by L y n n and E s p i n a s in 1959. j'2 T h e f r e q u e n t c o e x i s t e n c e of a s s o c i a t e d a n o m a l i e s in o t h e r s y s t e m s ( p a r t i c u l a r l y c a r d i o v a s c u l a r defects), as well as the p r e s e n c e of a n n u l a r pancreas, malrotation, and other midline a l i m e n t a r y d e f e c t s such as e s o p h a g e a l atresia and i m p e r f o r a t e anus, s u g g e s t the d u o d e n a l atresia o c c u r s r e l a t i v e l y e a r l y in i n t r a u t e r i n e life. A l t h o u g h intrinsic m u c o s a l atresia is f r e q u e n t l y o b s e r v e d in i n s t a n c e s of d u o d e n a l a t r e s i a , j e j u noileal a t r e s i a as a result of epithelial p l u g g i n g is u n c o m m o n . C l i n i c a l o b s e r v a t i o n s by S a n t u l l i and B l a n c and N i x o n noting bile p i g m e n t s , s q u a m e s , and l a n u g o hairs distal to a t r e t i c segments indicates that factors other than epithelial p l u g g i n g a r e involved. 3'4 In 1955, L u o w and B a r n a r d s u b j e c t e d dog fetuses to ligation of m e s e n t e r i c vessels and s t r a n g u l a t i o n o b s t r u c t i o n late in the c o u r s e of p r e g n a n c y . 5 T h e s e m a n i p u l a t i o n s r e s u l t e d in a v a r i e t y o f a t r e t i c c o n d i t i o n s s i m i l a r to those o b s e r v e d clinic a l l y in n e o n a t e s . T h e s e f i n d i n g s s t r o n g l y s u g g e s t e d t h a t m o s t j e j u n o i l e a l and colonic a t r e sias (exclusive of cases a s s o c i a t e d with i m p e r f o r a t e anus) w e r e the result of a late i n t r a u t e r i n e m e s e n t e r i c v a s c u l a r a c c i d e n t . L a b o r a t o r y invest i g a t i o n by C o u r t o i s , 6 S a n t u l l i a n d Blanc, 4 A b r a m s , 7 and K o g a et al. 8 c o n f i r m e d t h e s e o b s e r v a t i o n s in e x p e r i m e n t a l studies p e r f o r m e d on fetal rabbits, sheep, and dogs, r e s p e c t i v e l y . F r e q u e n t clinical i n s t a n c e s of intestinal a t r e s i a as a result of m e s e n t e r i c v a s c u l a r insults such as volvulus, i n t u s s u s c e p t i o n , i n t e r n a l hernia, and c o n s t r i c t i o n of the m e s e n t e r y in a t i g h t g a s t r o s chisis or omphalocele defect, have been reported.9 ~3 d e L o r i m i e r et al. noted e v i d e n c e of bowel i n f a r c t i o n in 4 2 % o f 619 c a s e s o f j e j u n o ileal atresia. ~4 N i x o n and T a w e s noted m a c r o scopic or m i c r o s c o p i c i n t r a u t e r i n e peritonitis in

372

61 of 127 patients with an obvious volvulus noted in 44.15 Luow recognized three types of jejunoileal atresia: Type I mucosal atresia, Type II the atretic ends connected by a band of fibrous tissue, and Type III the atretic ends separated by a V-shaped mesenteric defect. 16 This has been the classification used most extensively throughout the literature, particularly in the large reviews where Type III cases have been the most common variant. Little attention has been given to those cases of multiple atresias with foreshortened bowel and a string of sausage appearance, familial instances of atresia, and infants with "apple-peel" atresia. 17-19The latter cases present with jejunal atresia near the ligament of Treitz, foreshortened bowel, a large mesenteric gap defect, and the distal bowel precariously supplied in a retrograde fashion by anastomotic arcades from the ileocolic, right colic, or inferior mesenteric artery. Patients with this distinct variation of atresia appear to have a familial pattern, are often of low birthweight, and have a high mortality. Martin and Zerella recently proposed a new classification to include instances of multiple atresia and "apple-peel" atresia. 2~ We have modified this classification somewhat to retain the previous nomenclature of Luow as well as add "apple-peel" atresia as a special category of Type III (llIb) and consider multiple atresias as Type IV (Fig. 2). Under the newer classification, Type Ilia cases with a mesenteric gap defect still occurred most frequently in the present group of patients. Operative therapy in instances of intestinal atresia is related to the pathological findings and the specific set of circumstances encountered in each individual case. In instances of duodenal obstruction, side-to-side duodenojejunostomy has long been considered the procedure of choice in many centers. In contrast, we believe a direct duodenal operation is superior. The only anastomotic complications in the present report occurred in two patients with duodenojejunostomy. In both instances, late obstruction required revision to a duodenoduodenostomy. Rowe et al. 21 and Richardson and Martin 22 described transgastric catheter techniques to delineate instances of "windsock" duodenal web and suggested that duodenotomy and web excision was the most appropriate therapy. This proce-

GROSFELD, BALLANTINE, AND SHOEMAKER

dure was performed successfully in nine patients in this study. The remaining 19 patients underwent successful duodenoduodenostomy. In a recent review of 85 newborns with intrinsic duodenal atresia or stenosis, Stauffer and Irving observed that the highest long-term survival rate was achieved in patients with duodenoduodenostomyY Aubrespy et al. in a critical study of anastomoses commonly used for duodenal atresia, emphasized that duodenojejunostomy and gastrojejunostomy should be avoided. 24 They recommended an operative technique that reduces the size and remodels the proximal dilated duodenal segment followed by duodenoduodenostomy. They further emphasized the importance of dividing bands or adhesions related to malrotation as possible causes of recurrent obstruction. The survival rate was 84% in the present report and is an improvement when compared to the 50%-60% survival rates documented in the survey by the Surgical Section of The American Academy of Pediatrics conducted by Fonkalsrud et al. z5 and the large series reported by Stauffer and Irving23 (85 cases) and Aubrespy et al. 24 (82 cases) on the European continent. Although associated anomalies, prematurity, and an increased incidence of fatal pneumonia in patients with Down's Syndrome contribute heavily to mortality data, failure to recognize some of the complex operative factors (especially occlusive webs and associated malrotation, annular pancreas, situs inversus, and anterior portal vein) may also play a role. Rowe et al. 2~ and Richardson and Martin 22 have documented instances of double duodenal webs and the literature is full of reports documenting diagnosis of duodenal web at autopsy. The availability of neonatal intensive care facilities, improved recognition of the defect, careful preoperative preparation, a direct duodenal operation, and early detection of potentially correctable associated anomalies may improve the outlook for these patients. In regard to the operative treatment of jejunoileal atresia, appropriate credit should be given to L u o w , ~6 Nixon,4 and Benson 26 upon whose experience much of the current trends of operative therapy is based. They became aware that anastomoses involving the dilated blind proximal atretic segment often was associated with delayed function. They also observed that

INTESTINAL ATRESIA AND STENOSIS

the side-to-side anastomosis resulted in functional obstruction and subsequent development of the blind loop syndrome. Nixon reported that the change in the dilated proximal atretic segment consisted of smooth muscle hypertrophy and enlargement of bowel diameter. 27 This segment of bowel had ineffective peristalsis and failed to function at lower pressures following operation, deLorimier et al. 28 and Cloutier, 29 however, suggested that hyperplasia is the main change occurring in intestinal smooth muscle above a chronic obstruction. In instances of a chronic complete obstruction, such as jejunal atresia, hypertrophy may be so extreme that a state of decompensation is reached where even strong contractions never sufficiently close the intestinal lumen to cause an increase in pressure or to allow efficient propulsion at the normal inlet pressure. In cases of jejunal atresia, resection of the dilated proximal atretic segment back to the ligament of Treitz (Fig. 3A) or a reduction antimesenteric jejunoplasty (Fig. 3B) as advocated by Thomas, 3~followed by end-to-end anastomosis obviates these complications and usually results in a successful outcome. Wide proximal resection and anastomosis is also utilized for instances of mid-small bowel or ileal atresia as long as bowel length is adequate. Tapering enteroplasty may be advantageous in instances of ileal atresia associated with "short gut" and generalized distention of the entire proximal bowel. Although Benson 26 has suggested that an end-to-side ileal ascending colonic anastomosis is a reasonable alternative in instances of distal ileal atresia, it has the major disadvantage, however, of bypassing the ileocecal valve. In the present study, primary anastomosis was not attempted in cases of jejunoileal atresia associated with instances of intrauterine volvulus when the vascular integrity of the remaining small bowel was in question, in the presence of severe peritonitis, or with obvious meconium ileus. In these cases, resection of the atretic segments and an exteriorization procedure is recommended. The most expeditious procedure is a side-by-side (modified Mikulicz) doublebarrel enterostomy. This procedure is rapidly performed, avoids an intraperitoneal anastomosis, and allows the stomata to be easily evaluated for intestinal viability in the postoperative period. In addition, re-operation at a later date

373

requires only a limited target laparotomy to restore intestinal continuity by end-to-end anastomosis. It is of interest that in patients with atresia or stenosis associated with gastroschisis, both enterostomy and anastomosis were well tolerated despite the angry appearance of the bowel due to its antenatal exposure to amniotic fluid. Anastomotic suture lines remain intact even when residing in a dacron reinforced silo during staged abdominal closure. Similarly, enterostomy in these cases was not associated with an increased risk of infection in the presence of the prosthetic material. Amoury et al. suggested that primary anastomosis in gastroschisis patients with atresia is relatively safe and may be advisable. 3~ On the other hand, Boles et al. have successfully managed atresia patients with gastroschisis by initial enterostomy and subsequent closure as was done in three patients in the present study. 32 The most common cause of death in infants with jejunoileal atresia is infection related to pneumonia, peritonitis, or sepsis. Anastomotic leak or bowel dysfunction, respiratory distress, prematurity, and short-bowel syndrome contribute to the morbidity and mortality data. Previous reports indicate an increased mortality in jejunal cases (42%), multiple atresias (57%), "applepeel" atresias (71%), and when atresia is associated with meconium ileus (65%), meconium peritonitis (50%), and gastroschisis (66%). ~3'~4 Nixon and Tawes suggested the use of "risk" and "treatment" groups in order to critically evaluate survival and mortality data in 127 instances of jejunoileal atresia. '5 Patients were placed into three risk groups: Group A--infants > 5'/2 lb with no other abnormalities; Group B--infants weighing 4-5~/2 lb or having a moderately severe associated abnormality; and Group C--infants weighing under 4 lb and/or with severe abnormalities. Survival was 81% in Groups A and B, but only 32% in Group C babies. "Treatment" groups were divided into high jejunal, mid-small bowel, and terminal ileal atresias. Patients with high jejunal atresia in risk Group A or B had a 60% survival, but all their Group C babies died. Survival occurred in 82% of mid-small bowel atresias in risk Group A or B, but was only 32% in Group C. Survival for atresias involving the terminal ileum in infants with risk Group A or B was 100%, but dropped

374

GROSFELD, BALLANTINE, AND SHOEMAKER

to 50% for infants in risk Group C in that series. ~5 The four deaths in the present series were all in risk Group C patients, deLorimier et al. noted that wide proximal resection and endto-end anastomosis improved survival in jejunal atresia from 39%-66% but had little effect in the overall survival in cases of ileal atresia. ~4 The overall survival rate in the 619 patients surveyed by deLorimier and his colleagues in the Surgical Section of the American Academy of Pediatrics was 68%. Luow reported a 94% survival rate following wide proximal resection and a onelayer end-to-end anastomosis in 33 infants with jejunoileal atresia. ~6 There were 2 anastomotic leaks noted in that study and 2 deaths occurred in risk Group C infants. Martin and Zerella recently reported survival of 38 of 59 (65%) infants with jejunoileal atresia. 2~Their data was evaluated according to cases that occurred prior to and after 1968. A 50% mortality was observed prior to 1968 in sharp contrast to cases operated upon since 1968 in which all of the infants have survived with the exception of three with associated cystic fibrosis. They attributed their recent improvement in results to wide resection of the proximal atretic segment and nutritional support. 2~ Aggressive use of total parenteral nutrition as an adjunctive method of therapy has significantly improved and overall outlook for infants with jejunoileal atresia. Total parenteral nutrition prevents protein-calorie malnutrition, establishes positive nitrogen balance, allows growth, prevents starvation-induced immune deficient states, and allows for a relatively safe waiting period in instances of atresia with anastomotic dysfunction or prolonged adynamic ileus that may accompany cases of atresia associated with gastroschisis. Total parenteral nutrition has also been extremely effective in instances of shortbowel syndrome and in cases with temporary exteriorization procedures who have enterostomy dysfunction. In instances of massive bowel resection, total parenteral nutrition maintains the nutritional needs of the patient while allow-

ing appropriate healing and time for adaptive mechanisms to eventually become effective. Although 9 of 12 infants with "short-bowel" syndrome in the present report survived, such patients may continue to have significant problems following discharge from the hospital and should be closely monitored. While most infants with a 50% mid-small bowel resection will have a relatively normal growth and developmental pattern, others with more extensive resections may not. Infants with more distal resections, particularly those in whom the ileocecal valve is excised, are more subject to malabsorption (fat, bile sales, calcium, B12, magnesium), diarrhea, and increased bacterial proliferation in the small bowel. Long-term follow up with regard to growth and development in such patients is essential. The colon is an unusual site of intestinal atresia and stenosis. The fact that 5 of 6 patients had a Type Ilia atresia strongly supports an intrauterine vascular accident involving the mesentery of the transverse or sigmoid colon as an etiologic factor. One patient had a Type I atresia. Babies with colonic atresia are usually full-term and rarely have associated anomalies. Clinical presentation is characteristic of low bowel obstruction as distention, vomiting, and failure to pass meconium are frequent findings. Barium enema is usually diagnostic both of colonic atresia and stenosis. 33 There is some controversy regarding appropriate operative management. Some authors favor resection and primary anastomosis for instances of colon atresia that occur proximal to the splenic flexure and a colostomy for those cases occurring distal to this site. 34'35 Boles and associates, however, reported survival in 10 of 11 infants (4 with gastroschisis) managed by initial end colostomy and subsequent anastomosis. 32 All seven patients in the present report had preliminary end colostomy that promptly relieved obstruction and allowed for early feeding. Re-operation and anastomosis at age 3-6 mo was successful in each case.

REFERENCES

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INTESTINAL ATRESIA AND STENOSIS

5. Luow JH, Barnard CN: Congenital intestinal atresia-Observations on its origin. Lancet 2:1065, 1955 6. Courtois B: Les origines foetales des occlusions cong6nitales due gr~le dites par atr6sia. J Chir (Paris) 78:405, 1959 7. Abrams JS: Experimental intestinal atresia. Surgery 64:185, 1968 8. Koga Y, Hayashida Y, Ikeda K, et al: Intestinal atresia in fetal dogs produced by localized ligation of mesenteric vessels. J Pediatr Surg 10:949, 1975 9. Grosfeld JL, Clatworthy HW Jr: Intrauterine midgut strangulation in a gastroschisis defect. Surgery 67:519, 1970 10, Grosfeld JL, Clatworthy HW Jr: The nature of ileal atresia due to intrauterine intussusception. Arch Surg 100:714, 1970 11. Spencer R: The various patterns of intestinal atresia. Surgery 64:661, 1968 12. Vassy LE, Boles ET: iatrogenic ileal atresia secondary to clamping an occult omphalocele. J Pediatr Surg 10:799, 1975 13. Hays DM: Intestinal atresia and stenosis in Ravitch M (ed): Current Problems in Surgery. Chicago, Year Book Publishers, 1969 14. deLorimier AA, Fonkalsrud EW, Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65:819, 1969 15. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atresias. Surgery 69:41, 1971 16. Luow JH: Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery 62:940, 1967 17. Zerella JT, Martin LW: Jejunal atresia with absent mesentery and a helical ileum. Surgery 80:550, 1976 18. Blyth H, Dickson JAS: Apple-Peel syndrome. J Med Genet 6:275, 1969 19. Zwiren GT, Andrews HG, Ahmann P: Jejunal atresia with agenesis of the dorsal mesentery ("apple-peel small bowel"). J Pediat Surg 7:414, 1972 20. Martin LW, Zerella JT: Jejunoileal atresia: A proposed classification. J Pediatr Surg 11:399, 1976

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21. Rowe MI, Buckner D, Clatworthy HW Jr: Windsock web of the duodenum. Am J Surg 116:444, 1968 22. Richardson WR, Martin LW: Pitfalls in the surgical management of the incomplete duodenal diaphragm. J Pediat Surg 4:303, 1969 23. Stauffer UG, Irving I: Duodenal atresia and stenosis--Long-term results. Prog Pediatr Surg 10:49, 1977 24. Aubrespy P, Derlon S, Seriat-Gautier B: Congenital duodenal obstruction: A review of 82 cases. Prog Pediatr Surg 11:109, 1978 25. Fonkalsrud EW, deLorimier AA, Hays DM: Congenital atresia and stenosis of the duodenum. A Review Compiled from the Members of the Surgical Section of the American Academy of Pediatrics. Pediatrics 43:79, 1969 26. Benson CD, Lloyd JR, Smith JD: Resection and primary anastomosis in the management of stenosis and atresia of the jejunum and ileum. Pediatrics 26:265, 1960 27. Nixon HH: An experimental study of propulsion in isolated small intestine and applications to surgery in the newborn. Ann R Cotl Surg Engl 27:105, 1960 28. deLorimier AA, Norman DA, Gooding CA, et al: A model for the cinefluoroscopic and manometric study of chronic intestinal obstruction. J Pediatr Surg 8:785, 1973 29. Cloutier R: Intestinal smooth muscle response to chronic obstruction: Possible application in jejunoileal atresia. J Pediatr Surg 10:3, 1975 30. Thomas CG Jr: Jejunoplasty for correction of jejunal atresia. Surg Gynecol Obstet 129:545, 1969 31. Amoury RA, Ashcraft KW, Holder TM: Gastroschisis complicated by intestinal atresia. Surgery 82:373, 1977 32. Boles ET, Vassy LE, Ralston M: Atresia of the colon. J Pediat Surg 11:69, 1976 33. Bley WR, Franken EA: Roentgenology of colon atresia. Pediatr Radiol 1:105, 1973 34. Coran AG, Eraklis A J: Atresia of the colon. Surgery 65:828, 1969 35. Freeman NV: Congenital atresia and stenosis of the colon. Br J Surg 53:595, 1966